199例肥厚型心肌病患者的长期随访研究

Long term follow-up results of 199 patients with hypertrophic cardiomyopathy

目的探讨肥厚型心肌病(HCM)患者的长期预后及其死亡的相关危险因素。方法回顾性分析1999年6月至2006年3月期间收入院的234例 HCM 患者的病史资料,并对其中获得随访的199例患者的随访结果采用 SPSS 13.0软件包进行统计学处理。结果 234例患者中,199例(85%)获得随访,35例(15%)患者失访。平均随访时间为(31.7±22.6)个月,由确诊到随访结束时间中位数为35个月。随访中21例患者死亡,其中19例患者被确认为 HCM 相关性死亡,包括心脏骤停11例(57.9%)、心力衰竭死亡7例(36.8%)、脑卒中死亡1例,另有2例患者分别因意外事故和急性胰腺炎死亡。患者确诊后1、2、3、4、5年生存率分别为96.7%、94.7%、94.7%、93.6%、89.0%。单因素分析显示,男性、心功能Ⅲ及以上、心房颤动、持续或短阵室速、左心房增大、左心室流出道梗阻、HCM 家族史7个变量与 HCM 预后相关。而多因素分析显示,仅持续或短阵室速(RR=2.234,P<0.001)、心功能Ⅲ及以上(RR=1.964,P=0.003)是 HCM 的独立预后危险因素。心脑事件死亡患者中,超声心动图表现以 MaronⅢ型为多见(73.7%),仅1例患者表现为心尖肥厚型心肌病(5.2%)。对心脏骤停者和心力衰竭死亡者进行分析显示,7例(63.6%)心脏骤停者发生在60岁以下的患者中(P=0.12,但仅1例患者小于35岁),5例(71.4%)心力衰竭死亡者发生在60岁以上的患者中(P=0.22)。仅2例心脏骤停者和1例心力衰竭死亡者存在静息状态下左心室流出道压差。7例(63.6%)心脏骤停者和2例(28.6%)心力衰竭死亡者合并阵发性室速。心脏骤停者与心力衰竭死亡者的室壁厚度分别为(20.4±4.7)mm 和(22.7±6.3)mm。结论 HCM 患者的中长期预后相对较好,以心肌肥厚仅累及心尖部者预后最佳。持续或短阵室速、心功能Ⅲ级以上是发生 HCM 相关心脑血管不良事件的独立危险因素。心脏骤停可发生在各年龄段,对其的预防在年轻和中老年患者中同样重要。

Objective To investigate the associations among clinical characteristics and prognosis in patients with hypertrophic cardiomyopathy （ HCM ）. Methods Clinical and follow up data of 234 patients with HCM hospitalized in our institute from June, 1999 to March ,2006 were retrospectively analyzed. Results A total of 199 out of 234 patients（85% ）were followed up for （31.7 ± 22.6） months. Twenty-one patients died during follow-up, and HCM-related deaths occurred in 19 patients including 11 （57.9%） patients with cardiac arrest, 7（36. 8% ）patients with heart failure, 1 patient with stroke. The non-cardiac deaths were 1 accident death and 1 death due to acute pancreatitis. The survival rates of all followed up patients at 1, 2, 3, 4 and 5 years were 96.7%, 94. 7% , 94. 7%, 93.6% and 89.0%, respectively. Single risk factor analysis indicated that male gender, severe cardiac dysfunction, atrial fibrillation, sustained or non-sustained ventricular tachycardia （SVT or NSVT）, left atrial enlargement, left ventricular outflow-track （LVOT） obstruction, family history were correlated with poor prognosis. Muhifactor analysis showed SVT or NSVT （ RR = 2. 234, P 〈 0. 001 ）, NYHA class Ⅲ-Ⅳ （ RR = 1. 964, P = 0. 003 ） were independent risk factors for death. Among the cardio-cerebral death patients, echocardiography showed 14/19 （73.7%） Mason type Ⅲ and 1/19 （5.2%）apical myocardial hypertrophy. Conclusions A relative benign long-term prognosis was shown in this group of HCM patients. Patients with apical myocardial hypertrophy, SVT or NSVT, NYHA class Ⅲ-Ⅳ were facing increased risk of deaths.